guest ::
| Home > Publications Database > Reduced hnRNPA3 increases C9orf72 repeat RNA levels and dipeptide-repeat protein deposition. > print |
| Home > Publications Database > Reduced hnRNPA3 increases C9orf72 repeat RNA levels and dipeptide-repeat protein deposition. > print |
| 001 | 138773 | ||
| 005 | 20250325124919.0 | ||
| 024 | 7 | _ | |a 10.15252/embr.201541724 |2 doi |
| 024 | 7 | _ | |a pmid:27461252 |2 pmid |
| 024 | 7 | _ | |a pmc:PMC5007570 |2 pmc |
| 024 | 7 | _ | |a 1469-221X |2 ISSN |
| 024 | 7 | _ | |a 1469-3178 |2 ISSN |
| 024 | 7 | _ | |a altmetric:10091664 |2 altmetric |
| 037 | _ | _ | |a DZNE-2020-05095 |
| 041 | _ | _ | |a English |
| 082 | _ | _ | |a 570 |
| 100 | 1 | _ | |a Mori, Kohji |0 P:(DE-HGF)0 |b 0 |e Corresponding author |
| 245 | _ | _ | |a Reduced hnRNPA3 increases C9orf72 repeat RNA levels and dipeptide-repeat protein deposition. |
| 260 | _ | _ | |a Hoboken, NJ [u.a.] |c 2016 |b Wiley |
| 264 | _ | 1 | |3 online |2 Crossref |b EMBO |c 2016-07-26 |
| 264 | _ | 1 | |3 print |2 Crossref |b EMBO |c 2016-09-01 |
| 336 | 7 | _ | |a article |2 DRIVER |
| 336 | 7 | _ | |a Output Types/Journal article |2 DataCite |
| 336 | 7 | _ | |a Journal Article |b journal |m journal |0 PUB:(DE-HGF)16 |s 1742903319_13303 |2 PUB:(DE-HGF) |
| 336 | 7 | _ | |a ARTICLE |2 BibTeX |
| 336 | 7 | _ | |a JOURNAL_ARTICLE |2 ORCID |
| 336 | 7 | _ | |a Journal Article |0 0 |2 EndNote |
| 520 | _ | _ | |a Intronic hexanucleotide (G4C2) repeat expansions in C9orf72 are genetically associated with frontotemporal lobar degeneration (FTLD) and amyotrophic lateral sclerosis (ALS). The repeat RNA accumulates within RNA foci but is also translated into disease characterizing dipeptide repeat proteins (DPR). Repeat-dependent toxicity may affect nuclear import. hnRNPA3 is a heterogeneous nuclear ribonucleoprotein, which specifically binds to the G4C2 repeat RNA We now report that a reduction of nuclear hnRNPA3 leads to an increase of the repeat RNA as well as DPR production and deposition in primary neurons and a novel tissue culture model that reproduces features of the C9orf72 pathology. In fibroblasts derived from patients carrying extended C9orf72 repeats, nuclear RNA foci accumulated upon reduction of hnRNPA3. Neurons in the hippocampus of C9orf72 patients are frequently devoid of hnRNPA3. Reduced nuclear hnRNPA3 in the hippocampus of patients with extended C9orf72 repeats correlates with increased DPR deposition. Thus, reduced hnRNPA3 expression in C9orf72 cases leads to increased levels of the repeat RNA as well as enhanced production and deposition of DPR proteins and RNA foci. |
| 536 | _ | _ | |a 344 - Clinical and Health Care Research (POF3-344) |0 G:(DE-HGF)POF3-344 |c POF3-344 |f POF III |x 0 |
| 536 | _ | _ | |a 342 - Disease Mechanisms and Model Systems (POF3-342) |0 G:(DE-HGF)POF3-342 |c POF3-342 |f POF III |x 1 |
| 542 | _ | _ | |i 2016-07-26 |2 Crossref |u http://onlinelibrary.wiley.com/termsAndConditions#vor |
| 542 | _ | _ | |i 2016-07-26 |2 Crossref |u http://doi.wiley.com/10.1002/tdm_license_1.1 |
| 588 | _ | _ | |a Dataset connected to CrossRef, PubMed, |
| 650 | _ | 7 | |a C9orf72 Protein |2 NLM Chemicals |
| 650 | _ | 7 | |a C9orf72 protein, human |2 NLM Chemicals |
| 650 | _ | 7 | |a Dipeptides |2 NLM Chemicals |
| 650 | _ | 7 | |a HNRNPA3 protein, human |2 NLM Chemicals |
| 650 | _ | 7 | |a Heterogeneous-Nuclear Ribonucleoprotein Group A-B |2 NLM Chemicals |
| 650 | _ | 7 | |a Proteins |2 NLM Chemicals |
| 650 | _ | 7 | |a RNA, Messenger |2 NLM Chemicals |
| 650 | _ | 7 | |a RNA, Small Interfering |2 NLM Chemicals |
| 650 | _ | 2 | |a Animals |2 MeSH |
| 650 | _ | 2 | |a Brain: metabolism |2 MeSH |
| 650 | _ | 2 | |a C9orf72 Protein |2 MeSH |
| 650 | _ | 2 | |a Dipeptides: metabolism |2 MeSH |
| 650 | _ | 2 | |a Fibroblasts |2 MeSH |
| 650 | _ | 2 | |a Gene Expression Regulation |2 MeSH |
| 650 | _ | 2 | |a Gene Knockdown Techniques |2 MeSH |
| 650 | _ | 2 | |a HeLa Cells |2 MeSH |
| 650 | _ | 2 | |a Heterogeneous-Nuclear Ribonucleoprotein Group A-B: metabolism |2 MeSH |
| 650 | _ | 2 | |a Humans |2 MeSH |
| 650 | _ | 2 | |a Neurons: metabolism |2 MeSH |
| 650 | _ | 2 | |a Protein Binding |2 MeSH |
| 650 | _ | 2 | |a Protein Transport |2 MeSH |
| 650 | _ | 2 | |a Proteins: genetics |2 MeSH |
| 650 | _ | 2 | |a Pyramidal Cells: metabolism |2 MeSH |
| 650 | _ | 2 | |a RNA Transport |2 MeSH |
| 650 | _ | 2 | |a RNA, Messenger: genetics |2 MeSH |
| 650 | _ | 2 | |a RNA, Small Interfering: genetics |2 MeSH |
| 650 | _ | 2 | |a Rats |2 MeSH |
| 700 | 1 | _ | |a Nihei, Yoshihiro |0 P:(DE-HGF)0 |b 1 |
| 700 | 1 | _ | |a Arzberger, Thomas |0 P:(DE-2719)2811333 |b 2 |
| 700 | 1 | _ | |a Zhou, Qihui |0 P:(DE-2719)2811347 |b 3 |
| 700 | 1 | _ | |a Mackenzie, Ian R |0 P:(DE-HGF)0 |b 4 |
| 700 | 1 | _ | |a Hermann, Andreas |0 P:(DE-2719)2811732 |b 5 |
| 700 | 1 | _ | |a Hanisch, Frank |b 6 |
| 700 | 1 | _ | |a Degeneration, German Consortium for Frontotemporal Lobar |b 7 |
| 700 | 1 | _ | |a Alliance, Bavarian Brain Banking |b 8 |
| 700 | 1 | _ | |a Kamp, Frits |0 P:(DE-HGF)0 |b 9 |
| 700 | 1 | _ | |a Nuscher, Brigitte |0 P:(DE-HGF)0 |b 10 |
| 700 | 1 | _ | |a Orozco, Denise |0 P:(DE-2719)9000242 |b 11 |
| 700 | 1 | _ | |a Edbauer, Dieter |0 P:(DE-2719)2231621 |b 12 |
| 700 | 1 | _ | |a Haass, Christian |0 P:(DE-2719)2202037 |b 13 |e Last author |
| 773 | 1 | 8 | |a 10.15252/embr.201541724 |b : EMBO, 2016-07-26 |n 9 |p 1314-1325 |3 journal-article |2 Crossref |t EMBO reports |v 17 |y 2016 |x 1469-221X |
| 773 | _ | _ | |a 10.15252/embr.201541724 |g Vol. 17, no. 9, p. 1314 - 1325 |0 PERI:(DE-600)2025376-X |n 9 |q 17:9<1314 - 1325 |p 1314-1325 |t EMBO reports |v 17 |y 2016 |x 1469-221X |
| 856 | 7 | _ | |2 Pubmed Central |u http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5007570 |
| 856 | 4 | _ | |u https://pub.dzne.de/record/138773/files/DZNE-2020-05095_Restricted.pdf |
| 856 | 4 | _ | |u https://pub.dzne.de/record/138773/files/DZNE-2020-05095_Restricted.pdf?subformat=pdfa |x pdfa |
| 909 | C | O | |p VDB |o oai:pub.dzne.de:138773 |
| 910 | 1 | _ | |a Deutsches Zentrum für Neurodegenerative Erkrankungen |0 I:(DE-588)1065079516 |k DZNE |b 2 |6 P:(DE-2719)2811333 |
| 910 | 1 | _ | |a Deutsches Zentrum für Neurodegenerative Erkrankungen |0 I:(DE-588)1065079516 |k DZNE |b 3 |6 P:(DE-2719)2811347 |
| 910 | 1 | _ | |a Deutsches Zentrum für Neurodegenerative Erkrankungen |0 I:(DE-588)1065079516 |k DZNE |b 5 |6 P:(DE-2719)2811732 |
| 910 | 1 | _ | |a Deutsches Zentrum für Neurodegenerative Erkrankungen |0 I:(DE-588)1065079516 |k DZNE |b 11 |6 P:(DE-2719)9000242 |
| 910 | 1 | _ | |a Deutsches Zentrum für Neurodegenerative Erkrankungen |0 I:(DE-588)1065079516 |k DZNE |b 12 |6 P:(DE-2719)2231621 |
| 910 | 1 | _ | |a Deutsches Zentrum für Neurodegenerative Erkrankungen |0 I:(DE-588)1065079516 |k DZNE |b 13 |6 P:(DE-2719)2202037 |
| 913 | 1 | _ | |a DE-HGF |b Gesundheit |l Erkrankungen des Nervensystems |1 G:(DE-HGF)POF3-340 |0 G:(DE-HGF)POF3-344 |3 G:(DE-HGF)POF3 |2 G:(DE-HGF)POF3-300 |4 G:(DE-HGF)POF |v Clinical and Health Care Research |x 0 |
| 913 | 1 | _ | |a DE-HGF |b Gesundheit |l Erkrankungen des Nervensystems |1 G:(DE-HGF)POF3-340 |0 G:(DE-HGF)POF3-342 |3 G:(DE-HGF)POF3 |2 G:(DE-HGF)POF3-300 |4 G:(DE-HGF)POF |v Disease Mechanisms and Model Systems |x 1 |
| 914 | 1 | _ | |y 2016 |
| 915 | _ | _ | |a DBCoverage |0 StatID:(DE-HGF)0200 |2 StatID |b SCOPUS |
| 915 | _ | _ | |a DBCoverage |0 StatID:(DE-HGF)0300 |2 StatID |b Medline |
| 915 | _ | _ | |a DBCoverage |0 StatID:(DE-HGF)0310 |2 StatID |b NCBI Molecular Biology Database |
| 915 | _ | _ | |a DBCoverage |0 StatID:(DE-HGF)0320 |2 StatID |b PubMed Central |
| 915 | _ | _ | |a JCR |0 StatID:(DE-HGF)0100 |2 StatID |b EMBO REP : 2017 |
| 915 | _ | _ | |a DBCoverage |0 StatID:(DE-HGF)0199 |2 StatID |b Clarivate Analytics Master Journal List |
| 915 | _ | _ | |a WoS |0 StatID:(DE-HGF)0110 |2 StatID |b Science Citation Index |
| 915 | _ | _ | |a DBCoverage |0 StatID:(DE-HGF)0150 |2 StatID |b Web of Science Core Collection |
| 915 | _ | _ | |a WoS |0 StatID:(DE-HGF)0111 |2 StatID |b Science Citation Index Expanded |
| 915 | _ | _ | |a DBCoverage |0 StatID:(DE-HGF)1030 |2 StatID |b Current Contents - Life Sciences |
| 915 | _ | _ | |a DBCoverage |0 StatID:(DE-HGF)1050 |2 StatID |b BIOSIS Previews |
| 915 | _ | _ | |a IF >= 5 |0 StatID:(DE-HGF)9905 |2 StatID |b EMBO REP : 2017 |
| 920 | 1 | _ | |0 I:(DE-2719)1111016 |k AG Levin |l Clinical Neurodegeneration |x 0 |
| 920 | 1 | _ | |0 I:(DE-2719)1110004 |k AG Edbauer |l Cell Biology of Neurodegeneration |x 1 |
| 920 | 1 | _ | |0 I:(DE-2719)1110007 |k AG Haass |l Molecular Neurodegeneration |x 2 |
| 920 | 1 | _ | |0 I:(DE-2719)1510100 |k AG Teipel |l Clinical Dementia Research (Rostock /Greifswald) |x 3 |
| 920 | 1 | _ | |0 I:(DE-2719)5000080 |k AG Zhou |l Adaptive Immunity in Neurodegeneration |x 4 |
| 980 | _ | _ | |a journal |
| 980 | _ | _ | |a VDB |
| 980 | _ | _ | |a I:(DE-2719)1111016 |
| 980 | _ | _ | |a I:(DE-2719)1110004 |
| 980 | _ | _ | |a I:(DE-2719)1110007 |
| 980 | _ | _ | |a I:(DE-2719)1510100 |
| 980 | _ | _ | |a I:(DE-2719)5000080 |
| 980 | _ | _ | |a UNRESTRICTED |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1016/j.neuron.2013.02.004 |2 Crossref |o 10.1016/j.neuron.2013.02.004 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1007/s00401-013-1192-8 |2 Crossref |o 10.1007/s00401-013-1192-8 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1007/s00401-013-1189-3 |2 Crossref |o 10.1007/s00401-013-1189-3 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1126/science.1232927 |2 Crossref |o 10.1126/science.1232927 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1073/pnas.1315438110 |2 Crossref |o 10.1073/pnas.1315438110 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1007/s00401-013-1088-7 |2 Crossref |o 10.1007/s00401-013-1088-7 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1016/j.neuron.2013.10.015 |2 Crossref |o 10.1016/j.neuron.2013.10.015 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1016/j.celrep.2013.10.049 |2 Crossref |o 10.1016/j.celrep.2013.10.049 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1038/nature11922 |2 Crossref |o 10.1038/nature11922 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1002/j.1460-2075.1994.tb06883.x |2 Crossref |o 10.1002/j.1460-2075.1994.tb06883.x |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1038/nature14973 |2 Crossref |o 10.1038/nature14973 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1038/nature14974 |2 Crossref |o 10.1038/nature14974 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1038/nn.4085 |2 Crossref |o 10.1038/nn.4085 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1038/nature13124 |2 Crossref |o 10.1038/nature13124 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1126/science.1256800 |2 Crossref |o 10.1126/science.1256800 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1126/science.1254917 |2 Crossref |o 10.1126/science.1254917 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1007/s00401-014-1329-4 |2 Crossref |o 10.1007/s00401-014-1329-4 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1126/science.aaa9344 |2 Crossref |o 10.1126/science.aaa9344 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1016/j.neuron.2014.12.010 |2 Crossref |o 10.1016/j.neuron.2014.12.010 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1016/j.neuron.2015.09.015 |2 Crossref |o 10.1016/j.neuron.2015.09.015 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1038/embor.2012.90 |2 Crossref |o 10.1038/embor.2012.90 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1007/s00401-013-1181-y |2 Crossref |o 10.1007/s00401-013-1181-y |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1007/s00401-013-1200-z |2 Crossref |o 10.1007/s00401-013-1200-z |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1007/s00401-015-1450-z |2 Crossref |o 10.1007/s00401-015-1450-z |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1016/j.neuron.2011.09.011 |2 Crossref |o 10.1016/j.neuron.2011.09.011 |
| Library | Collection | CLSMajor | CLSMinor | Language | Author |
|---|